{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T21:01:20Z","timestamp":1793826080449,"version":"4.6.1"},"reference-count":54,"publisher":"Oxford University Press (OUP)","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neuropathol Exp Neurol"],"published-print":{"date-parts":[[2010,12]]},"DOI":"10.1097\/nen.0b013e3181ffe742","type":"journal-article","created":{"date-parts":[[2010,11,12]],"date-time":"2010-11-12T02:15:04Z","timestamp":1289528104000},"page":"1228-1246","source":"Crossref","is-referenced-by-count":63,"title":["Distinct Neuropathologic Phenotypes After Disrupting the Chloride Transport Proteins ClC-6 or ClC-7\/Ostm1"],"prefix":"10.1093","volume":"69","author":[{"given":"Sarah N.R.","family":"Pressey","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieran J.","family":"O'Donnell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Stauber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens C.","family":"Fuhrmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaana","family":"Tyynel\u00e4","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas J.","family":"Jentsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2010,12,1]]},"reference":[{"key":"2016030319035146000_69.12.1228.1","doi-asserted-by":"publisher","DOI":"10.1080\/10409230701829110"},{"key":"2016030319035146000_69.12.1228.2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2006.124719"},{"key":"2016030319035146000_69.12.1228.3","doi-asserted-by":"publisher","DOI":"10.1126\/science.1379744"},{"key":"2016030319035146000_69.12.1228.4","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00206-9"},{"key":"2016030319035146000_69.12.1228.5","doi-asserted-by":"publisher","DOI":"10.1038\/379445a0"},{"key":"2016030319035146000_69.12.1228.6","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-171"},{"key":"2016030319035146000_69.12.1228.7","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(95)01298-2"},{"key":"2016030319035146000_69.12.1228.8","doi-asserted-by":"publisher","DOI":"10.1038\/nature04535"},{"key":"2016030319035146000_69.12.1228.9","doi-asserted-by":"publisher","DOI":"10.1038\/nm842"},{"key":"2016030319035146000_69.12.1228.10","doi-asserted-by":"publisher","DOI":"10.1038\/sj.emboj.7600576"},{"key":"2016030319035146000_69.12.1228.11","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0606137103"},{"key":"2016030319035146000_69.12.1228.12","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2008.11.004"},{"key":"2016030319035146000_69.12.1228.13","doi-asserted-by":"crossref","first-page":"1Y13","DOI":"10.1093\/jnen\/62.1.1","article-title":"The neuronal ceroid-lipofuscinoses","volume":"62","author":"Haltia","year":"2003","journal-title":"J Neuropathol Exp Neurol"},{"key":"2016030319035146000_69.12.1228.14","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.bbadis.2006.05.006","article-title":"Molecular genetics of the NCLs\u2014 Status and perspectives","volume":"1762","author":"Siintola","year":"2006","journal-title":"Biochim Biophys Acta"},{"key":"2016030319035146000_69.12.1228.15","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.2004.tb00499.x"},{"key":"2016030319035146000_69.12.1228.16","doi-asserted-by":"publisher","DOI":"10.1096\/fj.09-130880"},{"key":"2016030319035146000_69.12.1228.17","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.25.2861"},{"key":"2016030319035146000_69.12.1228.18","doi-asserted-by":"publisher","DOI":"10.1359\/JBMR.040407"},{"key":"2016030319035146000_69.12.1228.19","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.2003.18.10.1740"},{"key":"2016030319035146000_69.12.1228.20","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.071015"},{"key":"2016030319035146000_69.12.1228.21","doi-asserted-by":"publisher","DOI":"10.1359\/jbmr.060403"},{"key":"2016030319035146000_69.12.1228.22","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2006.08.002"},{"key":"2016030319035146000_69.12.1228.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2006.09.001"},{"key":"2016030319035146000_69.12.1228.24","doi-asserted-by":"publisher","DOI":"10.1097\/nen.0b013e31815f3899"},{"key":"2016030319035146000_69.12.1228.25","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2009.02.001"},{"key":"2016030319035146000_69.12.1228.26","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2004.02.010"},{"key":"2016030319035146000_69.12.1228.27","doi-asserted-by":"publisher","DOI":"10.1177\/14.9.681"},{"key":"2016030319035146000_69.12.1228.28","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2004.07.030"},{"key":"2016030319035146000_69.12.1228.29","unstructured":"Paxinos G Franklin BJ . The Mouse Brain in Stereotaxic Coordinates. 2nd ed. San Diego, CA: Academic Press, 2001"},{"key":"2016030319035146000_69.12.1228.30","doi-asserted-by":"publisher","DOI":"10.1002\/ar.1092310411"},{"key":"2016030319035146000_69.12.1228.31","doi-asserted-by":"crossref","first-page":"2556","DOI":"10.1523\/JNEUROSCI.19-07-02556.1999","article-title":"Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: Evidence for partial response to insulin-like growth factor-1 treatment","volume":"19","author":"Cooper","year":"1999","journal-title":"J Neurosci"},{"key":"2016030319035146000_69.12.1228.32","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2005.05.018"},{"key":"2016030319035146000_69.12.1228.33","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2818.1987.tb02837.x"},{"key":"2016030319035146000_69.12.1228.34","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-0173(99)00007-7"},{"key":"2016030319035146000_69.12.1228.35","doi-asserted-by":"publisher","DOI":"10.1007\/s12038-008-0060-5"},{"key":"2016030319035146000_69.12.1228.36","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2005.01.025"},{"key":"2016030319035146000_69.12.1228.37","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2443.2002.00539.x"},{"key":"2016030319035146000_69.12.1228.38","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(01)00189-1"},{"key":"2016030319035146000_69.12.1228.39","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl107"},{"key":"2016030319035146000_69.12.1228.40","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410330408"},{"key":"2016030319035146000_69.12.1228.41","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980526)77:4<289::AID-AJMG8>3.0.CO;2-I"},{"key":"2016030319035146000_69.12.1228.42","doi-asserted-by":"publisher","DOI":"10.1086\/338190"},{"key":"2016030319035146000_69.12.1228.43","doi-asserted-by":"publisher","DOI":"10.1038\/13868"},{"key":"2016030319035146000_69.12.1228.44","doi-asserted-by":"crossref","first-page":"3599","DOI":"10.1002\/j.1460-2075.1995.tb00029.x","article-title":"Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells","volume":"14","author":"Saftig","year":"1995","journal-title":"EMBO J"},{"key":"2016030319035146000_69.12.1228.45","doi-asserted-by":"crossref","first-page":"6898","DOI":"10.1523\/JNEUROSCI.20-18-06898.2000","article-title":"Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons","volume":"20","author":"Koike","year":"2000","journal-title":"J Neurosci"},{"key":"2016030319035146000_69.12.1228.46","doi-asserted-by":"publisher","DOI":"10.1086\/504159"},{"key":"2016030319035146000_69.12.1228.47","doi-asserted-by":"publisher","DOI":"10.1080\/14992020600975279"},{"key":"2016030319035146000_69.12.1228.48","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e3283218807"},{"key":"2016030319035146000_69.12.1228.49","doi-asserted-by":"publisher","DOI":"10.1007\/s00347-008-1888-7"},{"key":"2016030319035146000_69.12.1228.50","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/111.1.27"},{"key":"2016030319035146000_69.12.1228.51","first-page":"151","article-title":"Morphologic studies on adult neuronal-ceroid lipofuscinosis (NCL)","volume":"1","author":"Goebel","year":"1982","journal-title":"Clin Neuropathol"},{"key":"2016030319035146000_69.12.1228.52","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1016\/j.bbadis.2006.05.007","article-title":"Functional biology of the neuronal ceroid lipofuscinoses (NCL) proteins","volume":"1762","author":"Kyttala","year":"2006","journal-title":"Biochim Biophys Acta"},{"key":"2016030319035146000_69.12.1228.53","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.125971"},{"key":"2016030319035146000_69.12.1228.54","doi-asserted-by":"publisher","DOI":"10.1126\/science.1188072"}],"container-title":["Journal of Neuropathology &amp; Experimental Neurology"],"original-title":[],"language":"en","deposited":{"date-parts":[[2019,6,6]],"date-time":"2019-06-06T03:04:12Z","timestamp":1559790252000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jnen\/article-lookup\/doi\/10.1097\/NEN.0b013e3181ffe742"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,12]]},"references-count":54,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2010,12,1]]},"published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1097\/NEN.0b013e3181ffe742"],"URL":"https:\/\/doi.org\/10.1097\/nen.0b013e3181ffe742","relation":{},"ISSN":["0022-3069","1554-6578"],"issn-type":[{"value":"0022-3069","type":"print"},{"value":"1554-6578","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,12]]}}}